Common pediatric esophageal disorders

Esophageal disorders in children can result in significant morbidity. The most common esophageal disorder seen in children is gastroesophageal reflux. Other common disorders affecting the esophagus include peptic esophageal strictures, esophageal atresia with or without tracheoesophageal fistula, caustic and foreign body ingestions, achalasia, and cricopharyngeal achalasia. We discuss what is currently known about these common pediatric esophageal disorders with regard to pathophysiology, clinical presentation, and diagnostic and treatment strategies.     

Treatment of advanced gastroesophageal reflux disease with Collis gastroplasty and Belsey partial fundoplication

OBJECTIVE: To examine the factors affecting outcome in patients with advanced gastroesophageal reflux disease. DESIGN: Retrospective analysis. SETTING: University tertiary referral center. PATIENTS: Thirty-seven patients with advanced gastroesophageal reflux disease and no previous antireflux surgery. INTERVENTIONS: Thirty patients underwent Collis gastroplasty for esophageal lengthening and Belsey partial fundoplication. Seven patients with esophageal stricture and global loss of esophageal body motility who underwent primary esophagectomy and reconstruction were used as a comparison group. OUTCOME MEASURES: Symptomatic outcome in all 37 patients was assessed by questionnaire at a median of 25 months (range, 5-156 months) after surgery. In a subset of 11 patients undergoing the Collis-Belsey procedure, outcome was measured using 24-hour pH and results of motility studies. RESULTS: The Collis-Belsey procedure was successful in relieving symptoms of gastroesophageal reflux in 21 (70%) of the 30 patients. The outcome was excellent or good in 16 (89%) of 18 patients who presented with symptoms other than dysphagia, but only in 5 (42%) of 12 patients with dysphagia (P = .01). The outcome was particularly poor if dysphagia was associated with a previously dilated esophageal stricture. Persistent or induced dysphagia was the reason for failure in all but 1 patient. Results of 24-hour esophageal pH studies were returned to normal in 8 (73%) of 11 patients undergoing postoperative evaluation. Contraction amplitudes in the distal esophagus and the prevalence of simultaneous contractions in these segments did not change after the operation. All 7 patients who underwent primary esophagectomy were classified as having an excellent or good outcome and were relieved of their reflux symptoms, including dysphagia. Six of these could eat 3 meals per day and enjoyed an unrestricted diet. CONCLUSIONS: The outcome of the Collis-Belsey procedure in patients with advanced gastroesophageal reflux disease without dysphagia is excellent. It is less so in patients with dysphagia as a preoperative symptom. Esophagectomy can provide a good outcome in patients who have a combination of dysphagia stricture and a profound loss of esophageal motility.     

Gastroesophageal reflux in children

BACKGROUND: Gastroesophageal reflux is common in small children. It is important to recognise that its natural history and management differ from reflux in adults. OBJECTIVE: This article will summarise the clinical presentation, pathophysiology, appropriate investigations and therapy of reflux in small children. DISCUSSION: A small amount of regurgitation is common in infancy, and is only pathological if it results in harm. In children, reflux-induced injury can result from either acid exposure, nutrient loss or respiratory complications. Recognised presentations include heartburn, oesophagitis, infant irritability, dysphagia, haematemesis, stricture, growth failure, aspiration, apnoea and pneumonia. Excessive crying and irritable behaviour in infancy can be due to a variety of causes, but there is a fashionable tendency to assume that gastroesophageal reflux is a major factor. Regurgitation can be a symptom of other conditions such as infection or metabolic disturbance. It is important to carefully evaluate any infant with distressed behaviour to exclude a significant medical cause. Prolonged oesophageal pH monitoring is a very useful means of linking episodes of reflux with putative symptoms. The severity of gastroesophageal reflux tends to improve with age in infants and this should be considered when advising appropriate therapy, especially fundoplication.     

Identification, diagnosis, and treatment of acid-related diseases in the elderly: implications for long-term care

Acid-related disorders such as peptic ulcer disease and gastroesophageal reflux disease occur frequently in the elderly and are associated with a high frequency of morbidity and mortality. The proton pump inhibitors lansoprazole and omeprazole produce faster rates of healing and greater symptomatic relief in patients with acid-related disorders than histamine2-receptor antagonists, are well tolerated, and are associated with few adverse events. Compared with omeprazole, which interacts with diazepam, warfarin, and phenytoin, lansoprazole produces only a minor increase in theophylline clearance. Proton pump inhibitors in combination with antibiotic therapy can eradicate Helicobacter pylori, the main risk factor in the recurrence of peptic ulcer disease, obviating the need for maintenance therapy. Long-term acid suppression with proton pump inhibitors may be necessary to prevent the recurrence of gastroesophageal reflux disease. The safety and efficacy profile of these agents makes them ideal for the treatment of acid-related diseases in elderly patients.     

Recent topics in the medical treatment of reflux esophagitis

Reflux esophagitis is one of the most common disorders of the upper gastrointestinal tract. It can lead to obstruction through stricture formation, in more severe form to bleeding through ulceration, and to cancer development through the association of Barrett's esophagus. The vast majority of esophagitis can be managed medically. Medical management is separated into two categories: (1) life style modification and (2) drug therapy. Drug therapy includes antacids, prokinetics, sucralfate, H2 receptor antagonists (H2-RAs) and proton pump inhibitors (PPIs). Among these, antisecretory therapy is the mainstay for the treatment. PPIs are shown to be superior to H2-RAs in healing of esophagitis and symptom relief. Recurrence, particularly of erosive esophagitis, is common without maintenance therapy. PPIs are also consistently superior to H2-RAs in maintenance of esophagitis healing. Interestingly, a recent report has suggested that curing Helicobacter pylori infection may provoke reflux esophagitis, raising the possibility that the gastroesophageal reflux diseases become more common in the future. Therefore, treatment strategy for reflux esophagitis needs to be re-established in terms of the future cost-effectiveness evaluation and quality-of-life assessments.     

Barrett's esophagus: a review

Complications of Barrett's esophagus include ulceration, stricture, hemorrhage, perforation, and the development of malignancy. Barrett's esophagus and adenocarcinoma may be diagnosed simultaneously and that gastroesophageal reflux symptoms may be absent in many cases. Although endoscopic surveillance is justified, no agreement on the frequency can be made. Survival of patients with adenocarcinoma in Barrett's esophagus depends on the stage at diagnosis.     

Gastroesophageal fundoplication for reflux in infants and children

Fifteen infants and young children with symptomatic gastroesophageal reflux underwent fundoplication during a 6 1/2-yr period. Standard barium esophagrams clearly demonstrated reflux in only 10 of the 15 patients; however, cine esophagrams indicated reflux in the remaining patients. Esophagoscopy with mucosal biopsy demonstrated esophagitis in 9 of the 10 patients in whom it was performed, and it is a very helpful diagnostic procedure. Esophageal manometry showed low sphincter pressures in each of 7 patients. Fundoplication was performed when there was (1) persistent reflux after a 3-wk hospital course of vigorous medical management, (2) failure to gain weight, (3) malnutrition, (4) recurrent aspiration, (5) esophagitis, or (6) stricture. Concomitant gastrostomy prevented the gas bloat syndrome in all patients. All strictures were successfully relieved by postoperative dilatation (average four per patient). Esophageal replacement is rarely indicated for strictures due to reflux in children. No deaths or major complications occurred following operation. Each of the patients has been relieved of clinical reflux, and each has gained weight more rapidly than preoperatively. Follow-up esophagrams on each of the patients show absence of reflux, and manometry shows the low esophageal sphincter pressure to be increased an average of 10 mm Hg above preoperative values. Since the results of Nissen fundoplication to correct reflux in infants and young children are highly satisfactory, and since the consequences of persistent reflux may be severe, a fairly aggressive approach should be taken in the management of symptomatic reflux.     

Oxidation of D(-)3-hydroxybutyrate administered to rats with extensive burns

We present 28 patients who underwent surgery after failed procedures due to gastroesophageal reflux with an asymptomatic period over 2.6 years. The most frequent complications resulted in recurrent reflux (86%) and peptic strictures (36%). An abdominal approach was used on all the patients. Reoperation, using the Collis-Nissen gastroplasty (19 cases), Nissen 360 degrees (3 cases), duodenal diversion with Roux-en-Y (3 cases) and Angelchick prothesis (3 cases) gave excellent or good results in 84% of the patients and poor in 4 cases. There was one operative death. All patients were referred for 24 hour pH-monitoring. Sphincter pressure and length of the distal esophageal sphincter were significantly increased over the preoperative values. Our results suggest that the Collis Nissen procedure is a suitable form of treatment for complicated forms of peptic esophagitis after the failed antireflux operations.     

Hiatus hernia and the respiratory tract

Hoarseness, asthma, and bronchitis are common but sometimes obscure manifestations of gastroesophageal reflux, the etiology of when respiratory symptoms predominate. In 300 consecutive patients who underwent surgical correction for gastroesophageal reflux, 129 (43%) had major respiratory complaints. Group 1 patients (82, 64%) were those referred for respiratory problems alone. In Group 2 (patients referred because of peptic complaints), 47 had associated respiratory problems in various combinations, including an additional 10 patients who had bronchiectasis. Treatment with appropriate surgical resection, in addition to antireflux procedures, was carried out in these people. Noticeable relief of respiratory symptoms was obtained in 96 (74%) of the 129 patients; 30 were improved and 2 were unchanged. Recurrent hiatus hernia or esophagitis was documented in 21 (7%) of the 300 patients.     

Circular myotomy and Belsey repair for acquired shortening of the oesophagus

Fourteen patients with shortening of the oesophagus due to chronic reflux oesophagitis have been treated by a combination of an oesophageal lengthening procedure with a standard anti-reflux repair. Their ages ranged from 18 to 78 years and eight were male. Thirteen patients had a reflux stricture (with additional penetrating ulcer in six) and one had a columnar-lined oesophagus. A complete circular myotomy was performed just below the level of the aortic arch, in combination with a Belsey Mark IV anti-reflux repair. There were no deaths post-operatively but one patient required re-operation for haemorrhage and one underwent oesophageal dilatation before discharge. All patients have now been followed up for over 5 years. Nine patients have required no further treatment for their oesophageal problems, two required two early dilatations each, two required multiple dilatations and one required oesophageal resection for stricture. We suggest that this combination merits further evaluation in the management of patients with short oesophagus due to reflux.     

Complications of gastroesophageal reflux disease. Esophagitis, acid laryngitis, and beyond

Gastroesophageal reflux disease is a common disorder that can result in various esophageal and extraesophageal complications. Reflux of gastric contents can cause esophageal mucosal abnormalities, such as ulcers and peptic strictures, as well as pulmonary and otolaryngologic symptoms, including reflux-induced asthma and acid laryngitis. Left untreated, some complications can lead to more severe disorders, such as esophageal adenocarcinoma that develops in patients with Barrett's esophagus. Accurate recognition of these diverse manifestations allows improved identification of patients at risk for reflux-related disorders and aids in proper evaluation and treatment.     

Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation

Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces. Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children. The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs. Bowel obstruction may be present at birth due to meconium ileus or meconium plug syndrome. Complications of meconium ileus include volvulus, small bowel atresia, perforation, and meconium peritonitis with abdominal calcifications. Older children with CF may present with bowel obstruction due to distal intestinal obstruction syndrome or colonic stricture, and tenacious intestinal residue may serve as a lead point for intussusception or cause recurrent rectal prolapse. Radiologic studies often demonstrate thickened intestinal mucosal folds in older children and uncommonly show colonic pneumatosis, peptic esophageal stricture due to gastroesophageal reflux, and duodenal ulcer. Appendicitis due to inspissated secretions is uncommon. Obstruction of ducts and ductules produces exocrine pancreatic insufficiency, pancreatitis, cholestasis, cholelithiasis, and cirrhosis with portal hypertension. On imaging studies, the pancreas is commonly small and largely replaced by fat, sometimes displays calcifications, and is rarely replaced by macrocysts. Radiologic features of hepatobiliary disease include an enlarged radiolucent liver from steatosis, gallstones, a shrunken nodular liver, splenomegaly, and portosystemic collateral vessels. With the improved survival of CF patients, an increased risk for developing gastrointestinal carcinomas has been established, many occurring as early as the 3rd decade.     

The endoscopic therapy of benign stenoses of the esophagus and cardioesophageal junction using Eder-Puestow instrumentation

The results of palliative endoscopic treatment by bougienage using Eder-Puestow instrumentation, performed in a limited contingent of patients presenting benign esophageal strictures, are analyzed. It is underscored that the method is readily carried out and effective, and seldom leads to noteworthy complications, such as esophageal perforations--one patient alone presenting cicatricial stricture out of the total of 42 cases given treatment with bougienage over a 10-year period. The commonest cause of benign strictures of the esophagus in the series being examined is reflux (peptic) esophagitis against the background of hiatus hernia.     

Parental smoking and infection with Helicobacter pylori among preschool children in southern Germany

BACKGROUND: Endoscopic examination (direct laryngoscopy and bronchoscopy) is the method of choice for diagnosis of respiratory symptoms of unknown cause in children. However, gastroesophageal reflux is being recognized increasingly often as a cause of pediatric respiratory symptoms and is difficult to diagnose on the basis of findings from direct laryngoscopy and bronchoscopy. In cases in which gastroesophageal reflux was included in the differential diagnosis, we additionally performed esophagoscopy with esophageal mucosal biopsies. OBJECTIVES: To determine the feasibility, safety, and efficacy of routinely performing esophageal biopsies during triple endoscopy in children. METHODS: Twenty-four children ranging in age from 2 weeks to 10 years were referred for airway evaluation. Under general anesthesia, children underwent direct laryngoscopy and bronchoscopy and esophagoscopy with mucosal biopsy. RESULTS: Esophageal mucosa biopsy specimens were quickly and safely obtained during endoscopic evaluation. There were no complications. Reflux esophagitis was present in 54% of biopsy specimens, as suggested by basal cell hyperplasia, papillary elongation, and/or inflammatory cell infiltrates. CONCLUSION: Gastroesophageal reflux is often difficult to diagnose in the pediatric population. When direct laryngoscopy and bronchoscopy is performed during examination of the child with airway symptoms, the addition of esophagoscopy with mucosal biopsies will safely and quickly provide data regarding the potential contribution of gastroesophageal reflux.     

Application of a 3D volume 19F MR imaging protocol for mapping oxygen tension (pO2) in perfluorocarbons at low field

Achalasia is a motility disorder of the esophagus characterized by the loss of inhibitory neurons in the distal esophagus. Although idiopathic in nature, autoimmune mechanisms have been proposed, and we set out to determine the presence of myenteric neuronal antibodies. We prospectively studied 18 patients with well-characterized achalasia (by clinical, x-ray, and manometric evidence), nine with gastroesophageal reflux disease, and analyzed the sera from 22 disease-free controls. Using double-label, indirect immunofluorescence techniques, rat esophageal and intestinal sections were double-labeled with sera (dilutions of 1:50 to 1:400) from the three groups and with neurofilament antibody to localize neurons. Seven of 18 achalasia patients had sera that stained the majority of neurons within plexi in the esophageal and intestinal sections, including both NADPH diaphorase (nitric oxide synthase) -positive and -negative neurons. None of the gastroesophageal reflux patients or the controls showed staining. Neuronal antibodies in achalasia provide an attractive hypothesis to explain this diffuse, possibly immune-based disorder.     

Barrett's esophagus: pathogenesis, epidemiology, functional abnormalities, malignant degeneration, and surgical management

Barrett's esophagus (i.e. columnar epithelial metaplasia in the distal esophagus) is an acquired condition that in most patients results from chronic gastroesophageal reflux. It is a disorder of the white male in the Western world with a prevalence of about 1/400 population. Due to the decreased sensitivity of the columnar epithelium to symptoms, Barrett's esophagus remains undiagnosed in the majority of patients. Gastroesophageal reflux disease in patients with Barrett's esophagus has a more severe character and is more frequently associated with complications as compared with reflux patients without columnar mucosa. This appears to be due to a combination of a mechanically defective lower esophageal sphincter, inefficient esophageal clearance function, and gastric acid hypersecretion. Excessive reflux of alkaline duodenal contents may be responsible for the development of complications (i.e., stricture, ulcer, and dysplasia). Therapy of benign Barrett's esophagus is directed towards treatment of the underlying reflux disease. Barrett's esophagus is associated with a 30- to 125-fold increased risk for adenocarcinoma of the esophagus. The reasons for the dramatic rise in the incidence of esophageal adenocarcinoma, which occurred during the past years, are unknown. High grade dysplasia in a patient with columnar mucosa is an ominous sign for malignant degeneration. Whether an esophagectomy should be performed in patients with high grade dysplasia remains controversial. Complete resection of the tumor and its lymphatic drainage is the procedure of choice in all patients with a resectable carcinoma who are fit for surgery. In patients with tumors located in the distal esophagus, this can be achieved by a transhiatal en-bloc esophagectomy and proximal gastrectomy. Early adenocarcinoma can be cured by this approach. The value of multimodality therapy in patients with advanced tumors needs to be shown in randomized prospective trials.     

A patient's perspective on the management of peptic esophageal stricture: experience and results in 113 consecutive cases

A retrospective analysis of 113 consecutive cases of benign esophageal stricture, all secondary to gastroesophageal reflux, 100 treated conservatively, 13 treated surgically, has been carried out in conjunction with a postal questionnaire of patients. Patients were requested to grade both their swallowing ability and the acceptability of their treatment. Of those responding to questionnaire, 88% of patients treated conservatively found their treatment acceptable or better, and 72% were left with either no or minimal restriction of diet. There was no correlation between either the total number or frequency of dilatations and the result achieved. Similarly, patient satisfaction appears largely independent of these variables. Doctors should be wary of taking recurrence of a stricture after initial dilatation as indicating a poor eventual outcome or a dissatisfied patient. There was no difference in terms of either the result or patient satisfaction between conservatively treated and surgically treated patients.     

Oesophageal acid clearance in patients with severe reflux oesophagitis

Previous studies examining oesophageal acid clearance have employed a variety of techniques to induce or simulate acid reflux. Clearance abnormalities have been deduced from abnormal standard motility studies, barium radiology or from 24-h pH recordings. In this study a 24-h pH and oesophageal motility recording system was used to study naturally occurring acid reflux episodes in control subjects and in two groups of patients with severe acid reflux disease (erosive oesophagitis and oesophageal stricture). Acid clearance was compared between the subject groups. Patients with oesophageal stricture were found to have poor oesophageal peristaltic ability and particularly poor oesophageal acid clearance. Those with erosive oesophagitis had normal peristaltic swallowing but abnormal acid clearance. This study has characterized, for the first time, the abnormalities in acid clearance during naturally occurring acid reflux episodes in patients with severe reflux disease. A more complete understanding of these clearance abnormalities could influence future medical and surgical strategies in the management of severe gastro-oesophageal reflux disease.     

Barrett's esophagus

The columnar replacement of squamous epithelium in the lower esophagus is the result of gastroesophageal reflux. Whether the squamous cells are replaced or undergo metaplasia is still conjectural. This neoepithelium is unstable in the presence of continued reflux and prone to complications of stricture, ulceration, and adenocarcinoma. Considerable evidence supports the hypothesis that duodenal contents play a role in the development of Barrett's esophagus and its complications. The increasing incidence of adenocarcinoma in Barrett's esophagus is of concern in the Western World. Surveillance programs in some centers have been successful in early diagnosis, and excellent survival periods have been reported following resection in these cases. Both medical and surgical antireflux treatment is successful in symptom relief, but even in the absence of symptoms, reflux may continue. Surgery offers better overall results than proton pump inhibition of gastric acid and has been more popular since less aggressive (minimally invasive) techniques have been popularized. Mucosal ablation and antireflux measures by medicine or surgery are still in the experimental stages but hold considerable promise for the future.     

Gastroesophageal reflux with protein-losing enteropathy and finger clubbing

Finger clubbing, protein-losing enteropathy, and iron deficiency were documented in three children with severe gastroesophageal reflux. One patient had Sandifer syndrome and the other two had the rumination syndrome. In each case, surgical repair of the gastroesophageal reflux resulted in immediate clearing of signs of the Sandifer syndrome, gastroesophageal reflux, and anemia and the return of serum protein levels to normal. There was definite regression of the finger clubbing during the ensuing year. It is suggested that finger clubbing, protein-losing enteropathy, Sandifer syndrome, and rumination be viewed as parts of an extended syndrome of unusual presentations of gastroesophageal reflux.